The collagen and glycosaminoglycan synthesis in corneal stromal and endothelial cell cultures derived from patients with granular, macular and lattice dystrophies of the cornea, keratoconus and mucopolysaccharidoses will be investigated together with normal controls. The response of each cell culture to various growth factors and lysosomal enzyme activities will also be examined. In addition, the amyloid produced in lattice dystrophy will be analyzed. Skin and conjunctival biopsies from affected patients and family members will also be used for comparative studies. We hope to detect and identify specific biochemical abnormalities associated with each disease. We further hope, with the cornea-conjunctiva skin model, to uncover any underlying systemic metabolic defect associated with each corneal dystrophy and keratoconus. The potential for skin biopsy diagnosis to predict future corneal disease is a possible extension of the present proposal.